Hepatopulmonary Syndrome and Venous Emboli Causing Intracerebral Hemorrhages After Liver Transplantation

Abrams, Gary A.; Rose, Kyle; Fallon, Michael B.; McGuire, Brendan M.; Bloomer, Joseph R.; Leeuwen, D. J. Van; Tutton, T.; Sellers, Marty T.; Eckhoff, Devin E.; Bynon, John S

doi:10.1097/00007890-199912150-00028

Cited by 42 publications

(18 citation statements)

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“…One-year survival rates in patients transplanted for HPS have been reported to be between 68% and 80%. 5 In addition, unique postoperative complications, including pulmonary hypertension, 11,12 embolic cerebral hemorrhage, 13 and postoperative deterioration in oxygenation have been described. Our prospective analysis confirms that mortality is increased after OLT in patients with HPS.…”

Section: Discussionmentioning

confidence: 99%

Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation

et al. 2003

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The hepatopulmonary syndrome (HPS) occurs in a subgroup of patients with cirrhosis and results from intrapulmonary vasodilatation, which may cause significant hypoxemia. Liver transplantation has emerged as a therapeutic option for patients with HPS based on retrospective case series and reports. However, morbidity and mortality appear to be increased after transplantation for HPS, and no prospective studies evaluating clinical features that may predict poor surgical outcome are available. Therefore, we prospectively evaluated the utility of the degree of hypoxemia, the arterial oxygen response to 100% oxygen administration, and the macroaggregated albumin (MAA) scan quantification of intrapulmonary shunting as predictors for outcome after liver transplantation. Our cohort consisted of 24 patients with cirrhosis and HPS who underwent liver transplantation over a 5-year period at 2 transplant centers who were followed at least 1 year after transplantation. All patients underwent preoperative evaluation for HPS with standardized methods. Seven patients (29%) died postoperatively, 5 of cardiorespiratory complications. All deaths occurred within 10 weeks after transplantation. A preoperative arterial oxygen tension (PaO 2 ) of < 50 mm Hg alone or in combination with a MAA shunt fraction > 20% were the strongest predictors of postoperative mortality. In conclusion, we found that mortality is increased after liver transplantation for HPS, particularly in patients with more severe hypoxemia and significant intrapulmonary shunting. Preoperative testing for the severity of HPS can be used to stratify patients according to the risk for postoperative mortality. (HEPATOLOGY 2003;37:192-197.)

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Section: Discussionmentioning

confidence: 99%

Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation

et al. 2003

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“…Spider angiomata are found in approximately one-third of patients with or without HPS. Neurological complications, including hemorrhage, stroke, and cerebral abscess formation, have been described in HPS, presumably related to passage of embolic material from the venous to systemic arterial circulations via IPVDs (30,45,51,55,56).…”

Section: Clinical Presentationmentioning

confidence: 99%

Pulmonary Vascular Complications of Liver Disease

Fritz

Fallon²,

Kawut

2013

Am J Respir Crit Care Med

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Hepatopulmonary syndrome and portopulmonary hypertension are two pulmonary vascular complications of liver disease. The pathophysiology underlying each disorder is distinct, but patients with either condition may be limited by dyspnea. A careful evaluation of concomitant symptoms, the physical examination, pulmonary function testing and arterial blood gas analysis, and echocardiographic, imaging, and hemodynamic studies is crucial to establishing (and distinguishing) these diagnoses. Our understanding of the pathobiology, natural history, and treatment of these disorders has advanced considerably over the past decade; however, the presence of either still increases the risk of morbidity and mortality in patients with underlying liver disease. There is no effective medical treatment for hepatopulmonary syndrome. Although liver transplantation can resolve hepatopulmonary syndrome, there appears to be worse survival even with transplantation. Liver transplantation poses a very high risk of death in those with significant portopulmonary hypertension, where targeted medical therapies may improve functional status and allow successful transplantation in a small number of select patients.Keywords: hepatopulmonary syndrome; hypertension; pulmonary; liver cirrhosis; pulmonary circulationThe pulmonary circulation may be affected by pathogenic processes arising within the liver and portal venous system. Reports of abnormalities of the pulmonary vasculature found in association with coexisting chronic liver disease were first published in the 1950s (1, 2). Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PoPH) are both associated with portal hypertension and/or liver disease and in simplest terms are related to excessive pulmonary microvascular dilation (in HPS) or vasoconstriction/vascular obstruction in pulmonary resistance vessels (in PoPH). The fact that these seemingly disparate conditions can occur within the same patient population (and even simultaneously or sequentially in the same patient) suggests the role of disease modifiers that determine the particular pulmonary vascular phenotype. These important pulmonary complications both commonly present with dyspnea, yet have unique pathophysiologies, diagnostic modalities, approaches to treatment, and distinct implications regarding liver transplantation (LT). A thorough understanding of the approach to evaluation and treatment of patients with cirrhosis and possible pulmonary vascular disease is imperative to minimize sequelae and to ensure appropriate management of the underlying liver disease.HEPATOPULMONARY SYNDROME Definition HPS is a disorder of altered gas exchange due to abnormal capillary dilatation and/or arteriovenous fistulae within the pulmonary vascular bed (3). The European Respiratory Society Task Force on Pulmonary-Hepatic Vascular Disorders has defined HPS by the presence of (1) Use of the A-a gradient criterion for abnormal gas exchange captures cases of HPS that would not otherwise meet the Pa O 2 criterion (e.g., due to cirrhosis-in...

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“…1.14. 15 Such prognostic studies may further the cause to identify earlier adverse trends in oxygenation and alter the priority for liver transplantation, especially in view of the potential for syndrome resolution. Liver transplant priority consideration in patients with HPS exists in the pediatric population (amended United Network for Organ Sharing Policy 3.6; appendix 3B, June 25, 1998), but has not been applied to adult listing criteria.…”

Section: Oltmentioning

confidence: 99%

Hepatopulmonary syndrome: Recent literature (1997 to 1999) and implications for liver transplantation

Krowka

2000

Liver Transpl

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ith the success of orthotopic liver transplanta-W tion (OLT) has come a renewed interest in hepatopulmonary syndrome (HPS) and pulmonary hypertension (PH), which have emerged as the most important pulmonary problems.'*2 Although a cause-andeffect understanding of specific mediators related to these vascular problems remains elusive, there is no question that the resolution of severe hypoxernia (in the case of HPS) and normalization of pulmonary hemodynamics (in the case of PH) may occur. However, it is equally clear that the severity of HPS and subsets of patients with PH (portopulmonary hypertension) may have grave prognostic implications, even if an otherwise successful OLT has been accomplished. This synopsis focuses on HPS, with particular emphasis on selected liver transplantation literature published within the last 2 to 3 years.

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Hepatopulmonary Syndrome and Venous Emboli Causing Intracerebral Hemorrhages After Liver Transplantation

Cited by 42 publications

References 9 publications

Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation

Prospective evaluation of outcomes and predictors of mortality in patients with hepatopulmonary syndrome undergoing liver transplantation

Pulmonary Vascular Complications of Liver Disease

Hepatopulmonary syndrome: Recent literature (1997 to 1999) and implications for liver transplantation

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